Carpal tunnel syndrome in the mucopolysaccharidoses and mucolipidoses

Title

Carpal tunnel syndrome in the mucopolysaccharidoses and mucolipidoses

Creator

Haddad F S; Jones D H A; Vellodi A; Kane N; Pitt M C

Publisher

Journal of Bone and Joint Surgery - Series B

Date

1997

Subject

tone and motor problems; MLIII; MPSI; MPSII; MPSIII; MPSVI; surgical interventions; physical interventions; surgery; physiotherapy; exercises; simultaneous tendon release

Description

Children with a mucopolysaccharidosis or mucolipidosis suffer progressive disability of the hands, particularly in relation to dysfunction of the median nerve. This is an increasing problem because bone-marrow transplantation has dramatically improved survival without apparently changing the musculoskeletal manifestations. We have reviewed 48 children with these syndromes who required carpal tunnel decompression, recording symptoms, signs, radiological, electrophysiological and operative findings, histology and upper-limb function. In these children the carpal tunnel syndrome differs from that seen in adults. Symptoms are rare but signs such as decreased sweating, pulp atrophy, thenar wasting and manual clumsiness are much more common. At operation, the flexor retinaculum was thickened and a mass of white tenosynovium engulfed the flexor tendons. Most patients had some definite nerve constriction with a thickened epineurium. Functional improvement was seen after early decompression, with some benefit from simultaneous tendon release. Regular physiotherapy helped to maintain increased hand movement. We describe our assessment protocol, the physiotherapy and operative regime and the standard functional review which helps to maximise function in the hands and upper limbs of these children.

Rights

Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).

Citation

Haddad F S; Jones D H A; Vellodi A; Kane N; Pitt M C, “Carpal tunnel syndrome in the mucopolysaccharidoses and mucolipidoses,” Pediatric Palliative Care Library, accessed December 6, 2021, https://pedpalascnetlibrary.omeka.net/items/show/16671.

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